Aortic dilatation resulting in chronic aortic regurgitation and complicated by aortic dissection in a patient with turner's syndrome

Goldberg, Stanley M.; Pizzarello, Roy A.; Goldman, Mitchell; Padmanabhan, Venkat

doi:10.1002/clc.4960070408

Cited by 12 publications

(2 citation statements)

References 5 publications

(11 reference statements)

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“…36 One individual had aortic regurgitation and left ventricular hypertrophy noted before dissection, without other structural abnormalities. 25 One patient had aortic regurgitation 31 and another had aortic stenosis 50 without coarctation or BAV. Sixteen individuals had HBP of whom 5 were not known to have structural cardiac malformations 19,34,38,42,51 .…”

Section: Resultsmentioning

confidence: 99%

Cardiovascular Malformations and Complications in Turner Syndrome

1998

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When the diagnosis of Turner syndrome is made, a screening echocardiogram should be obtained. Referral to a cardiologist first may be appropriate, but physical examination does not substitute for visualization. Individuals with and without evidence of structural cardiac malformations should be monitored for HBP on a lifelong basis. In the absence of structural cardiac malformations or HBP, the risk for aortic dissection appears small, and repeated echocardiography or magnetic resonance imaging to follow aortic root diameters does not appear to be warranted based on data currently available. Protocols for following patients with structural malformations need to be individualized, and wholesale recommendations have little merit. A longitudinal study using magnetic resonance imaging or cardiac echocardiography to establish normal parameters for aortic root diameters and to follow aortic root changes is needed.

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Section: Resultsmentioning

confidence: 99%

Cardiovascular Malformations and Complications in Turner Syndrome

1998

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“…(1978); Lie (1982); Slater et al . (1982); Price & Wilson (1983); Goldberg et al . (1984); Allen et al ., 1986; Lin et al ., 1986; Subramaniam (1989); Apostolopoulos et al .…”

Section: Discussionmentioning

confidence: 99%

Hypertension is a major risk factor for aortic root dilatation in women with Turner's syndrome

Elsheikh¹,

Casadei

Conway

et al. 2001

Clinical Endocrinology

152

101

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Women with Turner's syndrome (TS) have a threefold increase in mortality, primarily as a result of their cardiovascular complications. Recently, the risk of fatal aortic dissection has come to light as a major cause of mortality in women with TS. The aim of this study was to assess the prevalence of aortic root dilatation in a group of women with TS and to investigate the factors contributing to its development. Thirty-eight women with TS attending a dedicated adult Turner clinic were examined clinically and by M-mode and two-dimensional echocardiography on at least one occasion. Aortic root dilatation was defined as an aortic root diameter greater than the 95th centile for body surface area. Fasting serum lipid concentrations were measured in all women. Additionally, 18 subjects underwent noninvasive assessment of central arterial stiffness using applanation tonometry. Fifty percent of subjects were hypertensive and a similar number had an abnormal echocardiogram. A bicuspid aortic valve was present in 33% of subjects, 16 women (42%) had ascending aortic root dilatation. This was associated with a bicuspid aortic valve in four women and hypertension in 11. Two women had isolated aortic root dilatation. Aortic root diameter was significantly associated with systolic blood pressure (r = 0.5, P = 0.003) and left ventricular thickness (r = 0.5, P = 0.02). There was no association with serum lipids or arterial compliance. Structural cardiac abnormalities are present in up to 50% of women with Turner's syndrome. Aortic root dilatation is a significant risk in women with Turner's syndrome and is closely dependent on blood pressure. Aortic root dilatation does not appear to be related to atherosclerosis and is more likely to be due to a mesenchymal defect. Regular surveillance of the aortic root diameter is essential in all women with Turner's syndrome and hypertension should be treated aggressively when present in order to minimize the risk of potentially fatal aortic dissection.

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References

2003

The Natural and Modifed History of Congenital Heart Disease

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Aortic dilatation resulting in chronic aortic regurgitation and complicated by aortic dissection in a patient with turner's syndrome

Cited by 12 publications

References 5 publications

Cardiovascular Malformations and Complications in Turner Syndrome

Cardiovascular Malformations and Complications in Turner Syndrome

Hypertension is a major risk factor for aortic root dilatation in women with Turner's syndrome

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